2014 Fall
CE@SCO
COURSE 8
Lumps and Bumps:
Ocular Oncology
COPE Course 42486-SD
SCO HOMECOMING / FALL CE WEEKEND • OCTOBER 9-12, 2014
9/16/2014
Benign Eyelid Lesions
• Nodules
Eyelid and Orbital
“Lumps and Bumps”
Anne P. Rowland, M.D.
Oculoplastics and Reconstructive Surgeon
Nodules
Chalazion
Acute hordeolum
• Tumors
Molluscum contagiosum
• Actinic Keratosis
Xanthalasma
• Seborrheic Keratosis
• Papilloma
• Pyogenic granuloma
• Melanocytic Nevus
• Neurofibroma
• Cysts
• Keratoacanthoma
• Hidrocystoma
•
•
•
•
• Sebaceous cyst
• Cyst of Zeiss
Chalazion
• What Is It?
• Cyst within tarsal plate caused by inflammation of blocked
meibomian gland
• What Does It Look Like?
• Raised, round, painless, firm lesion typically on the upper eyelid
• Who Gets It?
• M=F, all ages
• Predisposing conditions: blepharitis, rosacea
 Medical Management
 Warm compresses
 Topical antibiotic/steroid ointment/drops
 Steroid injection
 Surgical Management
 Incision and drainage
 Excision
1
9/16/2014
External Hordoleum
• What Is It?
• Staphylococcus abscess of lash follicle and associated gland of Moll or Zeiss
• What Does It Look Like?
• Tender bump at lid margin with associated swelling
• Who Gets It?
• M=F, all ages
• Predisposing conditions: poor nutrition, poor hygeine, history of recurrent
infections
 Medical Management
 DO NOT forcefully rupture – may cause bacterial spread
 Topically or oral antibiotics
 Surgical Management
 Typically not necessary
 Excision after full course antibiotics
Molluscum Contagiosum
• What Is It?
• Viral induced lesion on the skin or mucous membranes
• May cause chronic follicular conjunctivitis or superficial keratitis
• What Does It Look Like?
• Painless, waxy umbilicated nodule
• Who Gets It?
• Children
• Patients with AIDS - multiple
 Medical Management
 OTC salicylic acid
 Tretinoin cream
 Requires several months of treatment and may cause
discomfort
 Surgical Management
Xanthalasma
• What Is It?
• Deposition of lipid or cholesterol beneath skin usually on or around eyelids
• What Does It Look Like?
• Sharply demarcated yellowish plaques; bilateral; medial
 Cryosurgery
 Curettage
2
9/16/2014
 Who Gets It?
 Elderly
Cysts
 Mediterranean or Asian descent
 Hypercholesterolemia
 Medical Management
 Deep TCA chemical peels
 Lasers
 Surgical Management
 Cryosurgery
Hidrocystoma
(Moll’s gland cyst)
Eyelid margin
Translucent
 Excision
Benign Tumors
Sebaceous Cyst
Often at medial
canthus
“Cheesy” contents
Cyst of Zeiss
Anterior lid margin
Opaque/white
Actinic Keratosis
• What Is It?
• Slow growing, pre-malignant keratinization of skin
• Results from excessive sun exposure
• What Does It Look Like?
• Flat, scaly, rough plaque;
may be multiple
• Rare on eyelid
 Who Gets It?
 Elderly
 Fair-skinned
 Medical Management
 None for eyelid
 Surgical Management
Seborrheic Keratosis
• What Is It?
• Slow growing, pre-malignant keratinization of skin
• Results from excessive sun exposure
• What Does It Look Like?
• Flat, greasy, often pigmented
• “Stuck-on” appearance
 Cryosurgery
 Excision
3
9/16/2014
 Who Gets It?
 Elderly
 Medical Management
 None
 Surgical Management
 Biopsy for definitive diagnosis
Papilloma
• What Is It?
• Proliferation of fibrovascular tissue covered by irregular keratinized
squamous epithelium
• May be caused by viral infection
(HPV)
• What Does It Look Like?
• “Skin tag”
• Pedunculated or broad base
 Who Gets It?
 Anyone
 Medical Management
 None
 Surgical Management
 Biopsy for definitive diagnosis
Pyogenic Granuloma
• What Is It?
• Overgrowth of vascular tissue
• What Does It Look Like?
• Fast-growing, pinkish lesion
• Pedunculated or flat based
• Who Gets It?
• History of recent surgery or trauma
 Medical Management
 Topical or intralesional steroid
 Surgical Management
 Excision
 Beware of recurrence
Melanocytic Nevus
• What Is It?
• Tumor composed of melanocytes; congenital or acquired
• What Does It Look Like?
• Junctional: Uniform brown macule or plaque
• Compound: Uniform, light to dark brown, raised papule
• Intradermal: Papillomatous with little to no pigment. Associated with
dilated vessels and protruding lashes
• Who Gets It?
• May become more pigmented in puberty
4
9/16/2014
Junctional
Intradermal
Compound
Neurofibroma
• What Is It?
• Abnormal proliferation of Swann cells and fibroblasts
• Plexiform type found on the eyelid
• What Does It Look Like?
Elevated
May be amelanotic
No malignant potential
Elevated
Usually pigmented
Low malignant
potential
Has components of
both intradermal and
junctional
• Large, “s-shaped” elevated lesion on upper eyelid
• “Bag of worms” texture
• Who Gets It?
• Solitary lesion in adults
• 25% associated with Neurofibromatosis – 1 (multiple)
 Surgical Management
 Excisional biopsy
 Medical Management
 None
 Surgical Management
 Excision
Keratoacanthoma
• What Is It?
• Uncommon pre-malignant proliferation of squamous epithelium
• What Does It Look Like?
• Fast growing, keratin-filled
crater with rolled edges
• May spontaneously involute
within 1 year
 Who Gets It?
 Elderly, fair-skinned
 Immunosuppressed
 Medical Management
 None
 Surgical Management
 Occasionally cryosurgery
Malignant Eyelid Lesions
• Basal cell carcinoma
• Squamous cell carcinoma
• Sebaceous cell carcinoma
• Melanoma
• Merkel cell carcinoma
• Kaposi sarcoma
 Excision
5
9/16/2014
Basal Cell Carcinoma
 Who Gets It?
 Elderly, fair-skinned
 Excessive sun exposure
• What Is It?
• Most common eyelid cancer
• Slow growling, locally destructive proliferation of basal cells
• What Does It Look Like?
• Usually on lower eyelid; destruction of normal architecture
 Treatment
 Biopsy
 Excision with 3-4mm clear margins
• Nodular type: pearl like, with dilated blood vessels on surface
• Ulcerative type: central ulcer with raised pearly edges
• Sclerosing type: lateral, hardened, infiltration beneath the
epidermis. May be confused with chronic blepharitis
Squamous Cell Carcinoma
 Elderly
 Fair skinned, un exposure, immunosuppressed
• What Is It?
• Aggressive cancer, car arise de novo or from AK or keratoacanthoma
• Can metastasize to local lymph node in 20%
• What Does It Look Like?
•
•
•
•
 Who Gets It?
Scaly with irregular boarders; may bleed
Nodular type: keratinized nodule; develops erosions and fissures
Ulcerating type: everted boarders with red, well defined base
Cutaneous horn: invasive growth underlies keratin horn
 Treatment
 Excision with 3-4mm clear margins
 +/- cryotherapy, radiation
 Can be fatal if untreated
Sebaceous Cell Carcinoma
• What Is It?
Lower Eyelid
Reconstruction After
Cancer Removal
• Slow growing cancer arising from meibomian glands, glands of Zeiss or
caruncle
• Usually on upper eyelid
• What Does It Look Like?
•
•
•
•
Can appear similar to chalazion or chronic blepharitis
May have yellowish material internally
Nodular type: hard, painless, immobile nodule; chalazion-like
Spreading type: thickened lid margin, loss of lashes; blepharitis-like
6
9/16/2014
 Who Gets It?
 Females, elderly (60’s – 70’s)
 Treatment
 Cryotherapy and surgical excision are the standard
treatments
 Recurrence is as high as 33%
 Mortality rate is 5-10%
 Who Gets It?
 Caucasians, sun exposure
 More advanced in dark-skinned people
 Increasing incidence in people in their 20’s
 Treatment
 Wide surgical excision with up to a 1 cm margin
Melanoma
• What Is It?
• Highly fatal cancer arising from melanocytes
• What Does It Look Like?
• Variable colors (blue, black);
50% are amelanotic
• Asymmetric, irregular with
indistinct boarders
• Destruction of local anatomy and loss of lashes
Merkel Cell Carcinoma
• What Is It?
• Rare, highly aggressive, rapidly growing neuroencocirne tumor
• What Does It Look Like?
• Red, purple or violet well-defined nodule
• Overlying skin is intact
 Local lymph node dissection if more than 1.5 mm deep
 Close follow-up
 Who Gets It?
 Caucasians, elderly (average age 75)
 Sun exposure, immunocompromised
 Treatment
 CT and/or MRI imaging used to evaluate systemic spread
(many have mets at time of diagnosis)
Kaposi’s Sarcoma
• What Is It?
• Vascular tumor caused by HHV-8
• What Does It Look Like?
• Erythematous or violaceous patch, path or nodule
 Excision with wide margins (3cm if possible)
 Chemotherapy and/or radiotherapy depending on
spread
 2 year mortality rate of 30-50%
7
9/16/2014
 Who Gets It?
Orbital Tumors
 Middle aged men
 Mediterranean or African descent
 AIDS
 Treatment
 Excision, cryotherapy, intralesional injections of
vinblastine, radiotherapy, topical immunotherapy
(Imiquod)
 Extensive disease requires chemo and
immunosuppression
Benign Orbital Tumors
Osteoma
adenoma)
• Bone
• Osteoma
• Fibrous dysplasia
• Well-delineated
•
•
•
•
•
• What Is It?
• Benign skeletal neoplasm of unknown etiology
• In the orbit, typically involves the frontal and ethmoid bones
• May cause pain, proptosis, decreased vision, or diplopia
Cavernous Hemangioma
Hemangiopericytoma
Dermoid cyst
Mucocele
ON tumors
• Diffuse
• Lymphangioma
• Benign reactive lymphoid
hyperplasia
• Lacrimal gland (pleomorphic
 What Does It Look Like?
 Radiographically, these tumors are well-circumscribed
with dense cortical sclerosis surrounding a radiolucent
nidus.
 Grossly, the lesion has a glistening, white to pink color
and is either smooth or with rounded protuberances
 Who Gets It?
Cavernous Hemangioma
• What Is It?
• Benign, noninfiltrative, slowly progressive vascular neoplasm
• Composed of endothelial-lined spaces surrounded by a well-delineated
fibrous capsule
• Most common benign orbital tumor in adults
• Typically located intraconally
 Younger patients, typically
found incidentally
 Treatment
 Excisional biopsy
8
9/16/2014
 What Does It Look Like?
 Presents as slowly progressive, painless proptosis
 May have EOM disturbance, induced hyperopia,
elevated IOP, choroidal folds or decreased vision
 On CT scan - well circumscribed, homogenous mass
slightly hyperdense to muscle, located intraconally.
 Who Gets It?
 Middle-aged adults, F>M
 Treatment
 May be observed as long as not comprising the eye
 Orbital excision indicated for growth, optic ON
compression, exposure keratopathy, or evidence of
vision loss.
 Who Gets It?
Dermoid Cyst
 Typically found in children
• What Is It?
• Congenital tumor consisting of keratinized epithelium and adnexal
structures (hair follicles, sweat glands, and sebaceous glands)
• What Does It Look Like?
• Egg-shaped, smooth, firm mass under the skin adjacent to bone
• CT scan - well-circumscribed lesion with a hyperdense wall and
hypodense contents. Bony remodeling is present in 85% of cases.
 Adults present with deeper, more posteriorly located
tumors
 Treatment
 If small, may be observed
 Complete excision – beware of inflammatory response if
ruptures or recurrence/abscess if incompletely removed
Mucocele
• What Is It?
Dermoid
Cyst
Excision
• Mucous or fluid-filled cyst arising from the ethmoid or frontal sinuses that
subsequently invades the orbit
• What Does It Look Like?
• Slowly progressive displacement of eye (may be axial or non-axial proptosis)
9
9/16/2014
 Who Gets It?
 Middle-age
 History of chronic sinus disease or
facial trauma
 Treatment
 MRI Drainage procedure- send fluid for culture
Lymphangioma
• What Is It?
• Diffusely infiltrating benign nonencapsulated vascular tumor
• What Does It Look Like?
• Fullness in the superior or nasal quandrant with acute proptosis after minor
head trauma or respiratory URI
• >50% affect anterior structures causing bluish discoloration of or blood
vessels within the eyelid skin.
• May bleed into itself causing cysts of blood, called chocolate-cysts
and cytology
 Surgery - orbitotomy and sinusectomy;
removal of as much of the cyst and its lining as possible
 Who Gets It?
 Any age
Pleomorphic Adenoma
• What Is It?
 Treatment
 If small, may be observed
• Neoplastic proliferation of epithelial cells in the lacrimal gland
• What Does It Look Like?
• Unilateral slowly progressive proptosis (inferomedial displacement)
 Complete excision – beware of inflammatory response if
ruptures or recurrence/abscess if incompletely removed
 Who Gets It?
 Middle age
 Treatment
 CT- solid (can be heterogenous), well defined, round or
oval, occasional calcification, and bony remodeling
 Complete surgical excision (incomplete removal can
result in recurrence or malignant transformation)
Malignant Orbital Tumors
• Bone
• Lymphoma
• Osteosarcoma
• Metastasis
• Well-delineated
• Metastasis
• Melanoma
• Diffuse
• Lymphoma
• Lacrimal gland
• Pleomorphic adenocarcinoma
• Adenoid cystic carcinoma
10
9/16/2014
Osteosarcoma
• What Is It?
• Very rare orbital tumor that forms osteoid
• Arises from soft tissue (NOT bone)
• What Does It Look Like?
• Rapidly growing, painless mass and/or proptosis
• CT – well-circumscribed calcified mass
 Who Gets It?
 Patients > 60 years old
 History of immunosuppressive or prior radiation
 Treatment
 Undefined (radical surgery, radiation, aggressive
chemotherapy)
 Poor prognosis, unless well-differentiated histiologically
 Who Gets It?
 History of cancer; however, the orbit is the first
presentation in 15%
 Treatment
 Diagnosis confirmed with fine needle aspiration
biopsies, serological studies, and molecular biology
techniques
Metastasis
• What Is It?
• Metastasis from breast carcinoma > melanoma > prostatic cancer
• What Does It Look Like?
• Proptosis, strabismus and vision loss
are the common clinical signs
• CT- solid enhancing mass located
within the orbital fat or enlargement of an
extraocular muscle
Primary Melanoma
• What Is It?
• Primary orbital melanomas arise from melanocytes (congenital ocular
melanosis, oculodermal melanosis or blue nevus)
• What Does It Look Like?
• Progressive painful ptosis,
visual blurring and scotomatas
 Multi-disciplinary and multiple modalities radiotherapy, chemotherapy, hormone therapy, surgery,
and immunotherapy
 Survival after diagnosis is 1.5 years on average,
independent of the histological type
11
9/16/2014
 Who Gets It?
 Caucasians, middle age
 Treatment
 Diagnosis with biopsy and immunophenotyping
 Determine primary status with full body imaging and
pathologic characteristics
 Exenteration +/- radiation and chemotherapy
Lymphoma
• What Is It?
• Non-Hodgkins lymphoma of the orbit
• May be associated with MALT lymphoma
and Chlamydia psittaci infection (usually the
result of exposure to infected birds and
household pets)
• What Does It Look Like?
• Typically superolateral painless mass causing palpable mas, exophthalmos,
ptosis, diplopia and abnormal ocular
movement
 Who Gets It?
 50-70 years old, M=F
 Treatment
 Chlamydia psittaci infection- antibiotic therapy reduce
the size of the tumor or possible cause remission
 Surgical biopsy / resection, radiotherapy and
chemotherapy are all used in various combinations
 65% 5-year relapse-free rate
Pleomorphic Adenocarcinoma
• What Is It?
• Malignant transformation of a pleomorphic adenoma (either spontaneously
or after incomplete excision)
• What Does It Look Like?
• Similar to pleomorphic adenoma
• EXCEPT: painful growth,
usually more rapid
 Systemic dissemination is only seen in 5-10% of cases
 Who Gets It?
 60-70 years old (10-20 years older than those with
pleomorphic adenoma)
 Treatment
 Surgical excision - lateral rhinotomy and medial
maxillectomy
Adenoid Cystic Carcinoma
• What Is It?
• What Does It Look Like?
• Painful rapidly growing mass in lacrimal gland
• CT: bony erosion, bone destruction and soft-tissue calcification
 Prognosis: survival rate correlates with the size, type,
and histologic grade

Undifferentiated carcinoma had the worst survival rate
(30%)

Polymorphous low-grade adenocarcinoma has the
highest survival rate (96%)
12
9/16/2014
 Who Gets It?
 M>F
 Younger age than other lacrimal malignancies (average
age is 41)
 Treatment
 Radical exenteration + radiation
 Prognosis poor

50% recurrence within
2 years

50% mortality rate within
1.5 years
The End!
(For those of you still
awake, any questions?)
13